P1-(2′-deoxycytidine 5′-)P4-(uridine 5′-)tetraphosphate, tetra-(alkali metal) salt, for treating cystic fibrosis

Abstract

The present invention provides a method of treating edematous retinal disorders. The method comprises administration of a pharmaceutical formulation comprising a hydrolysis-resistant P2Y receptor agonist to stimulate the removal of pathological extraneous fluid from the subretinal and retinal spaces and thereby reduce the accumulation of said fluid associated with retinal detachment and retinal edema. The P2Y receptor agonist can be administered with therapeutic and adjuvant agents commonly used to treat edematous retinal disorders. The present invention also provides a method of treating cystic fibrosis. The present invention further provides P 1 -(2′-deoxycytidine 5′-)P 4 -(uridine 5′-) tetraphosphate, tetra-(alkali metal) salts such as tetrasodium, tetralithium, tetrapotassium, and mixed (tetra-alkali metal) salts. The present further provides a pharmaceutical formulation comprising a P 1 -(2′-deoxycytidine 5′-)P 4 -(uridine 5′-) tetraphosphate, tetra-(alkali metal) salt, in a pharmaceutically acceptable carrier.

1. A method of treating cystic fibrosis in a subject in need thereof, said method comprising:
 administering to a subject suffering from cystic fibrosis P 1 -(2′-deoxycytidine 5′-)P 4 -(uridine 5′-)tetraphosphate, tetra-sodium salt, in an amount effective to treat cystic fibrosis. 
 
     
     
       2. A method of treating cystic fibrosis in a subject in need thereof, said method comprising:
 administering to a subject suffering from cystic fibrosis P 1 -(2′-deoxycytidine 5′-)P 4 -(uridine 5′-)tetraphosphate, tetra-(alkali metal) salt, in an amount effective to treat cystic fibrosis, wherein the tetra-(alkali metal) cations of said salt are tetralithium or tetrapotassium. 
 
     
     
       3. The method according to  claim 2 , wherein the tetra-(alkali metal) cations of said salt are tetralithium. 
     
     
       4. The method according to  claim 2 , wherein the tetra-(alkali metal) cations of said salt are tetrapotassium. 
     
     
       5. A method of treating cystic fibrosis in a subject in need thereof, said method comprising:
 administering to a subject suffering from cystic fibrosis P 1 -(2′-deoxycytidine 5′-)P 4 -(uridine 5′-)tetraphosphate, tetra-(alkali metal) salt, in an amount effective to treat cystic fibrosis, wherein the tetra-(alkali metal) cations of said salt are selected from the group consisting of: 
 monosodium/tripotassium, disodium/dipotassium, trisodium/monopotassium, 
 monopotassium/trilithium, dipotassium/dilithium, tripotassium/monolithium, 
 monosodium/trilithium, disodium/dilithium, trisodium/monolithium, 
 monosodium/monolithium/dipotassium, monosodium/dilithium/monopotassium and disodium/monolithium/monopotassium; and mixtures thereof.